title: Extraskeletal Ewing sarcomas in late adolescence and adults: a study of 37 patients.
abstract: BACKGROUND
  Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed.
  
  
  METHODS
  We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed.
  
  
  RESULTS
  The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS.
  
  
  CONCLUSIONS
  EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.
authors.affiliation: Department of Oncology, China PLA General Hospital, Beijing, China.
authors.forename: Hai-Tao
authors.initials: HT
authors.lastname: Tao
authors.forename: Yi
authors.initials: Y
authors.lastname: Hu
authors.forename: Jin-Liang
authors.initials: JL
authors.lastname: Wang
authors.forename: Yao
authors.initials: Y
authors.lastname: Cheng
authors.forename: Xin
authors.initials: X
authors.lastname: Zhang
authors.forename: Huan
authors.initials: H
authors.lastname: Wang
authors.forename: Su-Jie
authors.initials: SJ
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018943:Anthracyclines / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D006801:Humans; D008875:Middle Aged; D009362:Neoplasm Metastasis / Q000473:pathology; D009364:Neoplasm Recurrence, Local; D017599:Neuroectodermal Tumors / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery; D017671:Platinum Compounds / Q000627:therapeutic use*; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery; D015996:Survival Rate; D055815:Young Adult
chemical_list: D018943:Anthracyclines; D017671:Platinum Compounds